Established in 1988 by an Act signed into law by President Reagan, who himself was severely deaf, the National Institute on Deafness and other Communications Disorders (NIDCD) is one of the Institutes that comprise the National Institutes of Health (NIH). NIDCD is legislatively mandated to conduct and support biomedical and behavioral research and research training in the normal and disordered processes of hearing, balance, taste, smell, voice, speech, and language. The Institute also conducts and supports research and research training related to disease prevention and health promotion; addresses special biomedical and behavioral problems associated with people who have communication impairments or disorders; and supports efforts to create devices which substitute for lost and impaired sensory and communication function.
As part of their mission, the Institute periodically issues a roadmap, and we at The Hearing Blog are pleased to republish the Cochlear Implant and Hearing Aid Science Capsule excerpts from their 2012-2016 Strategic Plan, along with an augmented Executive Summary…
Science Capsule: Cochlear Implants
The development of the multi-channel cochlear implant has made it possible to restore the perception of sound to people who are profoundly deaf or severely hard of hearing (HoH). In contrast to hearing aids, which amplify sound, cochlear implants directly stimulate the auditory nerve.
Over the past two decades, NIDCD-supported research led to major advances in multi-electrode signal processing, as well as in understanding the benefits of early implantation in children and the possible benefits of implantation in both ears. Because of this research, we now know that children with hearing loss who receive a cochlear implant within the first two years of life will typically experience a smaller gap in language skills and will be more likely to succeed in mainstream classrooms.
According to the U.S. Food and Drug Administration (FDA), in December 2010, approximately 219,000 people worldwide have received cochlear implants, including approximately 42,600 adults and 28,400 children in the United States. Roughly 40 percent of children who are born profoundly deaf now receive a cochlear implant, which is a 25 percent increase from five years ago. The rise in cochlear implant use among eligible people between 2000 and 2010 exceeded the target set in the U.S. Department of Health and Human Services’ (HHS) Healthy People 2010 (a set of science-based 10-year national health objectives), and a new target is being developed for Healthy People 2020.
NIDCD-supported scientists continue to improve cochlear implant technology through the development of noise-reduction signal processing and innovative electrode designs. For example, insertion of traditional cochlear implant electrodes can damage hair cells throughout the cochlea, so researchers are investigating methods to preserve residual hearing in eligible individuals by implanting a shorter electrode array. In addition, animal studies are underway to assess the risks and benefits of a new electrode design that is positioned inside the auditory nerve, with the hope this will provide an improved sense of hearing in crowds and other social situations in which more than one person is speaking. NIDCD researchers continue studies with children who received cochlear implants at a young age to determine what factors contribute to successful language learning and subsequent academic performance. Continued research to assess how current users benefit from a cochlear implant in one ear, along with a cochlear implant or a hearing aid in the other ear, will help inform the design of the next generation of implants.
Science Capsule: Hearing Aids and Hearing Health Care
NIH- and NIDCD-supported research has driven the development of hearing aids from the first electronic hearing devices invented in the 1950s to the sophisticated digital devices available today. Innovative collaborations between the NIH, the Department of Veterans Affairs (VA), and the National Aeronautics and Space Administration (NASA) have significantly improved hearing aid technology over the past 20 years. In addition to amplifying sound, today’s hearing aids are better designed to address the challenges of understanding speech, localizing sound, and hearing in noisy environments.
Despite these advances, NIDCD-supported scientists are continuing to seek ways to improve hearing aid technology, hearing aid fitting strategies, and auditory rehabilitation programs to enrich the communication experience and quality of life for millions of Americans who have hearing loss. NIDCD-supported scientists are developing more effective methods to reduce sound distortion, improve sound localization, and combine hearing aid and cochlear implant technologies. For example, NIDCD-supported research on the tiny fly named Ormia ochracea provided a model for the development of a miniature directional microphone for hearing aids to help users focus on a single speaker in a noisy room.
Improving hearing health is an ongoing priority for NIDCD. An estimated 17 percent of all American adults and nearly half of adults ages 75 years and older have some form of hearing loss, yet only about 20 percent of those who could benefit from hearing aids actually use them. For the past two decades, the NIH and the VA have cosponsored biannual national and international meetings to facilitate information sharing among hearing aid technology researchers. In 2009, NIDCD convened a workshop titled “Accessible and Affordable Hearing Health Care for Adults with Mild to Moderate Hearing Loss,” that resulted in research recommendations and a series of NIDCD research initiatives to explore new approaches, assessment methods, and small business technologies to improve access to hearing health care for underserved individuals. In addition, increasing the rate of hearing aid usage was a HHS Healthy People 2010 goal and continues as a Healthy People 2020 goal. NIDCD is committed to pursuing research to understand and improve hearing health for all Americans.
Approximately one in six Americans will experience a communication disorder in his or her lifetime. Communication disorders affect hearing, balance, taste, smell, voice, speech, and language. For hearing and balance: estimates indicate that 36 million American adults report some degree of hearing loss; two to three out of 1,000 babies born in the United States each year have a detectable hearing loss; and almost eight million adults report a chronic problem with balance. For taste and smell: more than 200,000 people visit a physician for taste and smell disorders annually, and many more of these disorders go unreported. For voice, speech, and language: approximately 7.5 million people in the United States have trouble using their voices; by the first grade, roughly five percent of children have noticeable speech disorders, the majority of which have no known cause; and between six and eight million people in the United States have some form of language impairment.
Modern society depends on our ability to communicate with one another. While science and technology have greatly improved this capacity, life can be profoundly difficult for those with communication disorders. Such disorders can affect the emotional, social, educational, and cognitive development of an individual, and the cost of these disorders in terms of human suffering, unfulfilled potential, quality of life, and economic factors is incalculable.
The National Deafness and Other Communication Disorders Act of 1988 became Public Law 100-553 on October 28, 1988, establishing the National Institute on Deafness and Other Communication Disorders (NIDCD) within the National Institutes of Health (NIH). The mission of the NIDCD is to conduct and support biomedical research, behavioral research, and research training in the normal and disordered processes of hearing, balance, taste, smell, voice, speech, and language. The Institute also conducts and supports research and research training related to disease prevention and health promotion; addresses special biomedical and behavioral problems associated with people who have communication impairments or disorders; supports research evaluating approaches to the identification and treatment of communication disorders and patient outcomes; and supports efforts to create devices that substitute for lost and impaired sensory and communication function. NIDCD’s focus within this broad mission is to bring national attention to the disorders and dysfunctions of human communication and to advance biomedical and behavioral research to improve the lives of the millions of people with a communication disorder.
To accomplish this mission, NIDCD manages a broad portfolio of both basic and clinical research. The portfolio is organized into three program areas: Hearing and Balance; Taste and Smell; and Voice, Speech, and Language. The three program areas seek to answer fundamental scientific questions about normal function and disorders and to identify patient-oriented scientific discoveries for preventing, screening, diagnosing, and treating disorders of human communication.
Public Law 100-553 also requires NIDCD to prepare a plan to initiate, expand, intensify, and coordinate Institute activities concerning the disorders of hearing, balance, taste, smell, voice, speech, and language. NIDCD met this requirement by convening a task force of scientific experts in 1989 to prepare the first strategic plan, which guided the Institute over its first few years. NIDCD has continued to update or rewrite its Plan on a regular basis.
The NIDCD Strategic Plan (Plan) serves four purposes:
- It helps the Institute to prioritize its research investment;
- It informs the nation’s scientists of emerging areas of opportunity for research and provides them with guidance as they formulate their own research plans;
- It informs persons with communication disorders and their support organizations of research accomplishments and potential future breakthroughs;
- Finally, the Plan shares research progress and areas of future research opportunity with the public.
The goals listed in the NIDCD Strategic Plan were selected by experts as research areas that present the greatest scientific opportunities and public health needs over the next five years. The Plan is not a comprehensive list of all research areas that NIDCD is currently supporting or plans to support in the future. Basic and clinical research being supported by NIDCD will continue to be given high priority. The NIDCD is committed to supporting new, innovative, hypothesis-driven, meritorious research, which can lead to improving the health of individuals with communication disorders.
To develop the 2012-2016 Plan, NIDCD convened a series of working groups and solicited input from scientific experts, the National Deafness and Other Communication Disorders Advisory Council, NIDCD staff, and the public. In consultation with communication research scientists and the public, NIDCD identified four Priority Areas that have the potential to increase our understanding of the normal and disordered processes of hearing, balance, taste, smell, voice, speech, and language and to further our knowledge in human communication sciences.
Within each Priority Area, the Plan lists emerging research opportunities identified as goals. A summary of the research goals for the Hearing & Balance portion of NIDCD’s three program areas is listed below [Editors’ notes: Sections on Taste & Smell, and Voice, Speech & Language Research have been omitted; while each section of the Priority Areas has the full description from the Strategic Plan document added]:
Priority Area 1: Understanding Normal Function. Deepen our understanding of the mechanisms underlying normal function of the systems of human communication. By defining what is normal in both animal models and humans, we can better understand mechanisms of disease.
- Development of the Auditory and Vestibular System: Identify the molecules and genes involved in development of the peripheral and central auditory and vestibular pathways. Understand how auditory neurons recognize and establish tonotopic organization.
- Homeostasis and Microenvironment: Increase understanding of homeostasis in the inner ear (e.g., ionic composition and maintenance, inflammatory response and toxin elimination, blood-labyrinth barrier, microcirculation, hormonal and other control systems) and in the middle ear (e.g., gas exchange, fluid regulation, innate immunity, and gene expression).
- Mechanics: Expand knowledge of mechanics in the cochlea (e.g., interaction of hair cell membranes and sterocilia with supporting structures); in the middle ear (e.g., resolve important issues of middle ear mechanics, including tympanic membrane/ossicular coupling and the role of non-piston-like modes of stapes motion); and in the vestibular system (e.g., cupular and otolithic maintenance of posture and equilibrium).
- Sensory Cell Transduction: Identify the molecular constituents of hair cell transduction: nanomechanical properties, molecular motors in hair cell membranes and stereocilia, ion channels and pumps; and their integration for hair cell tuning and maintenance.
- Cochlear Amplification: Identify molecular determinants responsible for the biophysical traits that influence amplification, including the basis of its fast kinetics; delineate roles of stereociliar vs. somatic mechanisms in mammalian cochlear amplification; determine roles of amplification in low and high frequency regions of the cochlea; refine mathematical models of amplification and outer hair cell function.
- Functional Connectivity: Clarify how afferent and efferent neural circuits process auditory and vestibular peripheral input. Understand how coding schemes influence plasticity and enable attention, cognition, and stress. Incorporate advanced techniques of functional and structural neural imaging and connectivity, ranging from molecular to systems scale.
- Auditory System: Determine how sound detection, discrimination, and recognition interact with learning, memory, and attention as well as with vision, tactile sensation, and balance to better understand auditory perception in real-world listening environments.
- Vestibular System: Determine how vestibular, visual, and proprioceptive (the sensing of motion or position) systems interact to perceive space and motion and to maintain orientation.
Priority Area 2: Understanding Diseases and Disorders. Increase our knowledge of the mechanisms of diseases, disorders, and dysfunctions that impair human communication and health. Understanding mechanisms that underlie diseases and disorders is an important step in developing better prevention and treatment strategies.
- Epidemiology: Investigate natural history; genetic and environmental risk factors; racial, ethnic, and gender differences; and practical objective metrics for subpopulations to inform the development of evidence-based treatment strategies. Explore how complex comorbidities create differences in disease phenotypes and treatment outcomes.
- Inherited Disorders: Identify gene mutations responsible for congenital and age-related deficits, understand structural consequences of such mutations, and develop high-throughput platforms for testing individuals. Understand how specific mutations relate to the severity and progression of disease. Investigate protein function to inform better prevention and treatment strategies.
- Otitis Media: Improve understanding of susceptibility and pathogenesis related to genetics, prior upper respiratory infection, eustachian tube dysfunction and reflux, bacterial biofilms, polymicrobial infections, inflammatory dysregulation, and mucosal hyperplasia. Define immune pathways for effective middle ear protection by vaccines. Determine impact of vaccination on disease prevalence and infection by other microbes.
- Inflammatory and Autoimmune Responses of the Inner Ear: Identify and characterize first responders to injury in the inner ear. Determine how molecules and cells cross the blood-labyrinth barriers to initiate immune response and autoimmune disease. Identify genetic and epigenetic risk factors. Investigate innate and cognate immunity in resolution of OM.
- Tinnitus: Develop new animal models to understand the specific neural deficits responsible for tinnitus.
- Other Acquired Disorders: Improve understanding of the pathogenesis of noise-induced, traumatic, idiopathic, ototoxic, neurotoxic, metabolic, and non-hereditary degenerative auditory and vestibular dysfunction. Improve delineation of the multiple processes resulting in presbycusis. Relate molecular, cellular, and structural (e.g., temporal bone research) otopathology to the clinical progress of disease.
- Pathways and Damage: Determine how the peripheral and central auditory and vestibular pathways are reorganized following injury. Define the long-term changes resulting from early sensory loss. Identify molecular, genetic, and anatomical underpinnings of plasticity. Relate functional deficits to specific lesions in the pathways.
- Changes in Perception with Disease:
- Auditory System: Identify sources of variance contributing to large individual differences in response to similar intervention strategies among people with hearing loss. Improve understanding of the time course, sensitive periods, and complications of hearing loss
- Vestibular System: Understand how disease affects perception of motion and spatial orientation, including connections with limbic and autonomic systems.
Priority Area 3: Improving Diagnosis, Treatment, and Prevention. Develop, test, and improve diagnosis, treatment, and prevention of diseases, disorders, and dysfunctions of human communication and health. Diagnosis considers normal function and provides targets for prevention and treatment. Improvements in prevention and treatment lead to better outcomes with fewer side effects.
- Regeneration: Develop in vitro systems to identify genes and factors that promote regeneration of specific cellular phenotypes (e.g., hair cells, supporting cells, spiral ganglion neurons, cells of the stria vascularis); understand factors that regulate hair cell regeneration; and determine which genes and extracellular factors control cell-specific differentiation.
- Pharmacotherapeutics: Develop targeted delivery of viral vectors for gene therapy and site-specific, controlled, sustained molecular therapy for both developing and dysfunctional pathways. Develop therapies to improve neuronal stimulation, resist cell damage, and enhance cell repair.
- Tinnitus: Apply advanced imaging techniques to provide measures of changed neural activity in people with tinnitus. Identify pharmacologic agents to prevent tinnitus resulting from traumatic, ototoxic, degenerative, and other acquired disorders. Identify behavioral, pharmacological, surgical, and device-based treatments for improving tinnitus.
- Otitis Media: Develop polyvalent vaccines for polymicrobial middle ear infection. Develop new drug delivery systems to the middle ear to prevent development of, enhance innate immunity to, and speed recovery from inflammation. Develop therapies to prevent and treat biofilms.
- Interventions for Hearing Loss:
- Examine existing and develop better aural rehabilitation strategies. Investigate how aural rehabilitation strategies are affected by treating comorbid conditions that influence success, such as dementia, diabetes, osteogenesis imperfecta, and stress.
- Traditional (external) Hearing Aids: Improve device performance in background noise and other real-world settings.
- Cochlear Implants: Improve efficacy of bilateral implants, short electrode implants, and hybrid cochlear implant/hearing aids in the same or opposite ear in conjunction with auditory/aural rehabilitation, assistive electronic devices, sign language, in home and educational environments. Improve prediction of outcome and maintenance of outcome over time.
- Other Implants: Improve efficacy of partially and fully implantable middle ear devices, round window transducers, bone-anchored devices, ABI, and other brain implants.
- Interventions for Dizziness and Balance Disorders:
- Develop safer, better tolerated, and more effective pharmacological treatments for vertigo.
- Develop vestibular prosthetic devices and minimally invasive surgery for better control of imbalance and vertigo while preserving hearing and other functions.
- Develop improved behavioral approaches for the rehabilitation of chronic vestibulopathies.
- Develop improved methods of systematic diagnosis and delineation of subtypes of dizziness/vertigo in order to identify subpopulations that might respond best to targeted therapies.
- Understand post cochlear implantation dizziness and the connection with vestibular migraines.
- Hearing Disorders: Develop metrics that better define functional hearing and communication abilities under real-world listening conditions; differentiate clinical subtypes of hearing disorders; identify early pathology in its preclinical stage; provide better measures of performance, communication skills, and disease-specific quality of life instruments for cochlear implant users; and improve assessment of the perception of, and reaction to, tinnitus.
- Balance Disorders: Develop metrics for the perception of equilibrium, dizziness, vertigo, and spatial disorientation with emphasis on relationships among disequilibrium, emotional disabilities, and cognitive disabilities.
- Identify common data elements to improve communication among scientists and clinicians across different specialties.
- Management of Older Adults: Improve hearing loss management, including screening, treatment, and rehabilitation. Define the underserved population of older adults for hearing health care. Determine if early access to hearing health care changes health outcomes later in life. Develop and evaluate the effectiveness of screening methods. Reduce risk of falls in older adults due to imbalance. Develop assistive balance aids and training programs to augment stability and posture in the elderly.
- Clinical Trials and Other Clinical Research Studies: Develop and implement infrastructure to identify 1) investigators with expertise in epidemiology, clinical trials, data registry, and other clinical research and 2) academic- and community-based clinical practice settings with geographic, racial, and ethnic diversity in order to facilitate rigorous, cost-effective clinical research and maximize human subjects protections.
- Emerging Technologies (including Bioengineering, Nanotechnology, and Neural Prostheses): Capitalize on emerging scientific advances and technologies from nanoscience, biomedical engineering, and other areas to improve treatments and develop novel devices that support impaired function.
- Training: Promote the cross training of basic scientists, clinician scientists, and physician scientists to facilitate the development of interdisciplinary research teams and to stimulate translational research.
- Priority Area 4: Improving Outcomes for Human Communication. Accelerate the translation of research discoveries into practice; increase access to health care; and enhance the delivery, quality, and effectiveness of care to improve personal and public health. Scientifically validated prevention and treatment models will lead to better personal and public health only if they are translated effectively into routine practice.
- Hearing Health Care: Identify factors that influence a person’s motivation and perceived need for hearing health care. Examine the impact of organization, financing, and management of health care services on the delivery, cost, access to, and outcomes of services. Develop innovative delivery systems, including telehealth and the Internet, to increase awareness, access, and affordability. Identify cost-effective approaches for diagnosis and treatment.
- Comparative Effectiveness Research and Evidence-Based Medicine: Through clinical trials and epidemiological comparative effectiveness research, identify best treatments for a given medical condition for a defined set of individuals. Develop and use clinical registries, clinical data networks, and other forms of electronic health data to inform the conscientious, explicit, and judicious use of current best evidence in making decisions about hearing health care options.
- Implementation and Dissemination Research: Investigate effective implementation of “best practices” among health care providers to translate advances into routine community practice. Increase the effective dissemination of health information to the public to promote healthy behaviors.
- Community-Based Participation in Research: Promote community-based research to identify factors that influence outcomes for people with hearing and balance disorders in diverse real-world settings. Engage deaf and HoH individuals in community-based research to aid in developing behavioral interventions to improve their quality of life. Develop methods to address communication disorders in diverse populations, considering variations in care and practice settings.